Randomized controlled trial comparing the effectiveness of 308-nm excimer laser alone or in combination with topical hydrocortisone 17-butyrate cream in the treatment of vitiligo of the face and neck.

BACKGROUND: Vitiligo is a pigmentary disorder which may have disfiguring consequences. Its treatment remains a challenge. OBJECTIVES: We designed a parallel-group randomized controlled trial to compare the effectiveness of 308-nm excimer laser alone or in combination with topical hydrocortisone 17-butyrate cream in patients with vitiligo unresponsive to previous treatment with topical steroids or narrow-band ultraviolet (UV) B phototherapy. METHODS: Consecutive patients aged 18-75 years with nonsegmental vitiligo localized on the face and/or neck lacking response to previous conventional treatment were eligible. In total, 84 patients (44 women and 40 men, mean age 44 years) were randomized to 308-nm excimer laser phototherapy twice weekly alone or in combination with topical hydrocortisone 17-butyrate cream twice daily for three periods of 3 weeks followed by a 1-week steroid-free interval. The primary outcome was a reduction of at least 75% of the overall lesional areas as judged by automatic image analysis on reflected UV photographs, conducted blind to treatment assignment, at 12 weeks compared with baseline. Secondary outcomes were clearance, and improvements on Physician's Global Assessment (PGA) and Skindex-29 scores. RESULTS: A total of 76 (90%) patients completed the study. In an intention-to-treat analysis, seven [16.6%; 95% confidence interval (CI) 5.3-27.8%] patients in the excimer monotherapy arm and 18 (42.8%; 95% CI 27.8-57.8%) in the combination arm showed > or = 75% reduction of vitiligo lesions at 12 weeks (chi(2) test 6.89, P = 0.0087). Clearance was observed in two (4.7%; 95% CI 1.6-11.2%) and nine (21.4%; 95% CI 9.0-33.8%) patients, respectively (Fisher's exact test P = 0.04). A significant difference also emerged for PGA scores, while no difference was documented for Skindex-29. CONCLUSIONS: Recalcitrant vitiligo of the face and neck may benefit from the combination of excimer laser phototherapy with topical hydrocortisone 17-butyrate cream.

Autoimmune C1 inhibitor deficiency and angioedema.

C1 inhibitor (C1-INH) deficiency results in bouts of mucocutaneous edema and may be inherited (hereditary angioedema) or acquired (acquired angioedema). The syndrome of acquired angioedema, characterized by the adult onset of angioedema and by the lack of evidence of inheritance of the disease, may be associated with lymphoproliferative or other malignant diseases (type I) or with the presence of autoantibodies to C1-INH (type II); this is a rare variant form of C1-INH deficiency with angioedema. We report here a case of acquired C1-INH deficiency with angioedema, hypotension and abdominal discomfort observed in a 71-year-old man in whom complement abnormalities and autoantibodies against C1-INH have been observed and who was classified as having an autoimmune C1-INH deficiency. From the therapeutic point of view after resolution of the acute attacks, high doses of tranexamic acid have been able, at first, to decrease the frequency and the severity of the symptoms, and subsequently to provide a long symptom-free time.

Number and distribution of melanocytic nevi in individuals with a history of childhood leukemia.

BACKGROUND: An increased number of melanocytic nevi at the termination of chemotherapy has been documented in children with hematologic malignancies. The persistence of the increased number of nevi over time and the relationship with personal (e.g. phenotype) and disease related variables remain to be explored. METHODS: One hundred Italian patients diagnosed as having acute lymphatic or myeloid leukemia, after 1975, were recruited and compared with a group of 100 control individuals drawn from friend of the enrolled patients. Information regarding lifetime sun exposure, phenotypic characteristics, and number of nevi was collected by experienced dermatologists. Counts of nevi were expressed both as totals and as counts per unit of body surface area ("density"). Multiple linear regression analysis was employed to control for potentially confounding factors when comparing patients and controls. RESULTS: The patients and controls were fairly comparable in terms of constitutional characteristics, but the patients had a significantly higher number and density of nevi > or = 2 mm or larger in diameter. In addition, patients had a greater number of large nevi ( > or = 6 mm in greatest dimension), and of nevi in unusual areas, such as the palms and soles. Differences in nevus density between patients and controls were notably maintained in the older age group ( > 12 years). None of the disease-related factors analyzed (e.g. treatment protocol and radiotherapy), appeared to be significantly correlated with nevus density. CONCLUSIONS: Patients with a history of childhood leukemia have a sustained increase in their nevus density. A fairly convincing body of evidence indicates that a large number of melanocytic nevi is the strongest risk factor for melanoma. Therefore, the utility of periodic skin examination of these should be considered.

Cutaneous malignant melanoma appearing during photochemotherapy of mycosis fungoides.

We report a case of malignant melanoma that appeared in a 56-year-old man with mycosis fungoides (stage Ia) during treatment with PUVA. The cumulative UVA dose was 1,177 J/cm2. The pigmented lesion was removed and PUVA therapy discontinued. Histological examination revealed a superficial spreading malignant melanoma (1.77 mm thick, Clark level IV). The delayed-type cutaneous hypersensitivity was studied. The presence of a second malignancy after mycosis fungoides and PUVA therapy may have been coincidental. Nevertheless, this case suggests that the immunosuppression induced by mycosis fungoides and by PUVA therapy might be a pathogenetic factor in the development of malignant melanoma.

A comparative trial of desferrioxamine and hydroxychloroquine for treatment of porphyria cutanea tarda in alcoholic patients.

Forty male alcoholic patients with porphyria cutanea tarda (PCT) were randomly assigned to 2 groups of 20. The 1st group received desferrioxamine (30 mg/kg body weight/day for 1 week every 3 months) whereas the latter was given hydroxychloroquine (200 mg twice/wk orally). Alcohol abstinence was advised for all patients. Improvement of cutaneous signs was evident after 6 months in hydroxychloroquine-treated subjects and after 12 months in desferrioxamine-treated subjects. At the end of the 1-year clinical trial, significant decreases of serum iron and ferritin were found in all patients, irrespective of the therapy. Urinary total porphyrins were reduced significantly in both groups, but the drop was significantly more evident in hydroxychloroquine- than in desferrioxamine-treated subjects. After 1 year of therapy, 4 desferrioxamine-treated patients vs 16 hydroxychloroquine-treated subjects acquired a normal urinary porphyrin pattern. These results indicate that hydroxychloroquine is more effective than desferrioxamine in inducing clinical and biochemical remission of PCT. Accordingly, hydroxychloroquine should be the preferred alternative to phlebotomy, if the latter is contraindicated.

Mycosis fungoides. Peripheral T cell subsets defined by specific monoclonal antibodies.

Peripheral blood lymphocytes from 16 mycosis fungoides (MF) patients were studied using OKT series monoclonal antihuman T cell antibodies. The percentage of OKT3+ cells was in normal range for all MF patients compared with controls; the percentage of OKT4+ cells was significantly increased (p less than 0.002) in MF patients versus controls; the percentage of OKT8+ and OKT11+ cells in the MF group did not differ from controls. The OKT4+ cell expansion was apparently not dependent from the clinical stage of disease. These findings suggest that in MF patients there is a circulating OKT4+ cell expansion and, indirectly, that MF could be regarded as a helper T cell neoplasm.